Soft Tissue Sarcoma, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology

Mehren, Margaret von; Kane, John M.; Agulnik, Mark; Bui, Marilyn M.; Carr‐Ascher, Janai R.; Choy, Edwin; Connelly, Mary; Dry, Sarah M.; Ganjoo, Kristen N.; Gonzalez, Ricardo J.; Holder, Ashley M.; Homsi, Jade; Keedy, Vicki L.; Kelly, Ciara M.; Kim, Edward; Liebner, David A.; McCarter, Martin D.; McGarry, Sean V.; Mesko, Nathan W.; Meyer, Christian F.; Pappo, Alberto S.; Parkes, Amanda; Petersen, Ivy A.; Pollack, Seth M.; Poppe, Matthew M.; Riedel, Richard F.; Schuetze, Scott M.; Shabason, Jacob E.; Sicklick, Jason K.; Spraker, Matthew B.; Zimel, Melissa N.; Hang, Lisa E; Sundar, Hema; Bergman, Mary Anne

doi:10.6004/jnccn.2022.0035

Cited by 587 publications

(173 citation statements)

References 134 publications

(109 reference statements)

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“…and National Comprehensive Cancer Network (NCCN) guidelines, wide surgical margin resection and radiotherapy are needed [26,27] . However, the use of adjuvant or neo-adjuvant radiotherapy has largely gone understudied, since EMC has been considered for years a radiotherapy-resistant tumor [28] .…”

Section: Discussionmentioning

confidence: 99%

Primary extraskeletal myxoid chondrosarcoma of ovary with novel KIT mutation: A case report and literature review

Liu

Xiong

Peng

et al. 2022

Preprint

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Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant mesenchymal neoplasm of uncertain differentiation. Despite its name, this tumor type has no relationship to cartilage. Most tumors arise in deep soft tissue of the proximal extremities and limb girdles. The KIT mutation of this tumor is extremely rare and the ovary location as primary location has not been reported yet before. Case presentation: We present a case of primary EMC of ovary with novel KIT mutation of a 54-year-old female. To the best of our knowledge, we report the first primary EMC of ovary and the fourth case with KIT mutation. Conclusions With this case study we try to make a better understanding of tumor, lessen the diagnostic confusion by highlighting its rare location, and more importantly present substantial molecular data, which might help in providing more therapeutic opportunities in the future.

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Section: Discussionmentioning

confidence: 99%

Primary extraskeletal myxoid chondrosarcoma of ovary with novel KIT mutation: A case report and literature review

Liu

Xiong

Peng

et al. 2022

Preprint

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“…Therefore, the standard care of RMS is based on multiagent chemotherapy (CHT), followed by radiotherapy (RT) or concomitant CHT and RT. Additional CHT can be recommended depending on the prognostic group [ 30 , 31 ]. According to the North American approach, CHT is based on vincristine, actinomycin-D, and cyclophosphamide (VAC) [ 31 ].…”

Section: Introduction To Rhabdomyosarcomamentioning

confidence: 99%

“…Additional CHT can be recommended depending on the prognostic group [ 30 , 31 ]. According to the North American approach, CHT is based on vincristine, actinomycin-D, and cyclophosphamide (VAC) [ 31 ]. Alternating VAC to vincristine and irinotecan (VI) for patients with an intermediate-risk disease has been shown to give similar results but less toxicity [ 32 ].…”

Section: Introduction To Rhabdomyosarcomamentioning

confidence: 99%

Translational Implications for Radiosensitizing Strategies in Rhabdomyosarcoma

Pomella

Porrazzo

Cassandri

et al. 2022

IJMS

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Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence that includes FP-RMS, harboring the fusion oncoprotein PAX3/7-FOXO1 and FN-RMS, often mutant in the RAS pathway. Risk stratifications of RMS patients determine different prognostic groups and related therapeutic treatment. Current multimodal therapeutic strategies involve surgery, chemotherapy (CHT) and radiotherapy (RT), but despite the deeper knowledge of response mechanisms underpinning CHT treatment and the technological improvements that characterize RT, local failures and recurrence frequently occur. This review sums up the RMS classification and the management of RMS patients, with special attention to RT treatment and possible radiosensitizing strategies for RMS tumors. Indeed, RMS radioresistance is a clinical problem and further studies aimed at dissecting radioresistant molecular mechanisms are needed to identify specific targets to hit, thus improving RT-induced cytotoxicity.

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“…The conventional first-line treatment for advanced STS is anthracycline-based chemotherapy, which has a response rate of less than 20% and a median progression-free survival (PFS) of less than 6 months ( Seddon et al, 2017 ; Tap et al, 2017 ; Gamboa et al, 2020 ). Gemcitabine-based regimen is often considered as second-line treatment after anthracycline, with an expected median PFS similar to that of anthracyclines ( von Mehren et al, 2022 ). Multi-target tyrosine kinase inhibitors (TKIs) have also been shown to be effective against selective STS although they have a lower median PFS than anthracycline-based and gemcitabine-based regimens ( Kyriazoglou et al, 2022 ).…”

Section: Introductionmentioning

confidence: 99%

Efficacy and safety of sintilimab plus doxorubicin in advanced soft tissue sarcoma: A single-arm, phase II trial

et al. 2022

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Background: Chemoimmunotherapy is safe and efficacious in treating many types of malignant tumors. However, clinical data demonstrating the effect of this combination treatment in patients with metastatic soft tissue sarcoma (STS) are currently limited. This study evaluated the safety and efficacy of a programmed cell death protein 1 (PD-1) inhibitor plus doxorubicin in patients with advanced STS who failed previous systemic therapy.Methods: This was a single-center, single-arm, open-label phase II trial. Patients with unresectable or metastatic STS who had previously failed systemic therapy were enrolled. Patients received up to six cycles of doxorubicin and sintilimab (a PD-1 inhibitor), while sintilimab treatment continued for up to 2 years. Primary outcomes were objective response rate (ORR) and safety. Univariate Cox proportional hazards model was used to analyze the relationship between clinicopathological parameters and progression-free survival (PFS).Results: A total of 38 patients (20 men and 18 women) were enrolled in this study. The overall ORR was 39.5%, disease control rate was 71.1%, and the median PFS was 4.5 months [95% confidence interval (CI), 3.0–8.5 months]. The adverse events (AEs) associated with the combined treatment were mild, manageable, and well-tolerated. The most common grade 3 or higher AEs were hematologic, including leukopenia (21.1%), anemia (18.4%), and thrombocytopenia (18.4%). Patients with undifferentiated pleomorphic sarcoma (UPS) or dedifferentiated liposarcoma had a significantly longer PFS than those with other pathological subtypes [hazard ratio (HR) = 0.42, 95% CI 0.21–0.83; p = 0.013]. There was no significant difference in the median PFS between patients who had previously received anthracycline-based chemotherapy and those who had not (HR = 0.74, 95% CI 0.34–1.58, p = 0.43).Conclusion: Sintilimab plus doxorubicin is a safe and promising treatment for patients with advanced STS who have failed previous systemic therapy (including anthracycline-based chemotherapy). The efficacy of this combination therapy in UPS and dedifferentiated liposarcoma is superior to that in other sarcomas.Clinical Trial Registration:https://www.chictr.org.cn, registration number: ChiCTR1900027009.

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Soft Tissue Sarcoma, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology

Cited by 587 publications

References 134 publications

Primary extraskeletal myxoid chondrosarcoma of ovary with novel KIT mutation: A case report and literature review

Primary extraskeletal myxoid chondrosarcoma of ovary with novel KIT mutation: A case report and literature review

Translational Implications for Radiosensitizing Strategies in Rhabdomyosarcoma

Efficacy and safety of sintilimab plus doxorubicin in advanced soft tissue sarcoma: A single-arm, phase II trial

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